Santos Xiomara M, Bercaw-Pratt Jennifer L, Yee Donald L, Dietrich Jennifer E
Department of Obstetrics and Gynecology, Baylor College of Medicine, Houston, Texas, USA.
J Pediatr Adolesc Gynecol. 2011 Apr;24(2):e35-8. doi: 10.1016/j.jpag.2010.11.004. Epub 2010 Dec 28.
Although von Willebrand disease is the most common inherited bleeding disorder, platelet function disorders are less well recognized as a cause of bleeding. We report a case of menorrhagia caused by an unsuspected platelet secretion defect.
A 13-year-old Asian female, with unknown family history, presented with menorrhagia not responsive to intravenous conjugated estrogens, requiring transfusion of 7 units of packed red blood cells. Initial screening tests for bleeding disorders were normal; however, due to high clinical suspicion, further specific testing with platelet aggregometry was performed, which revealed a platelet secretion defect.
The prevalence of platelet secretion defects in adolescents with menorrhagia is unknown, but may be higher than currently recognized. When screening tests are normal, yet suspicion remains high for an underlying hemostatic disorder, platelet aggregometry must be performed.
尽管血管性血友病是最常见的遗传性出血性疾病，但血小板功能障碍作为出血原因却较少被认识。我们报告一例由未被怀疑的血小板分泌缺陷引起的月经过多病例。
一名13岁的亚洲女性，家族史不详，出现月经过多，对静脉注射结合雌激素无反应，需要输注7单位浓缩红细胞。最初的出血性疾病筛查试验正常；然而，由于临床高度怀疑，进行了进一步的血小板聚集试验特异性检测，结果显示存在血小板分泌缺陷。
月经过多青少年中血小板分泌缺陷的患病率尚不清楚，但可能高于目前的认识水平。当筛查试验正常但对潜在的止血障碍仍高度怀疑时，必须进行血小板聚集试验。
